Dobronravov V A, Smirnov A V
Research Institute of Nephrology, I.P. Pavlov First Saint Petersburg State Medical University of the Ministry of Health of the Russian Federation, Saint Petersburg, Russia.
Ter Arkh. 2018 Dec 30;90(12):39-47. doi: 10.26442/00403660.2018.12.000007.
Analysis of etiology, clinical and morphological manifestations, approaches to therapy and prognosis of membranoproliferative glomerulonephritis (MPGN).
Cases of MPGN were retrospectively identified in the period 2000-2017 with subsequent analysis of etiology, clinical data and morphology (including deposits of immunoglobulins (Ig) and C3 complement fractions). The achievement of complete and partial remissions (PR, CR), overall survival, progression (by composite endpoint: decrease in the estimated GFR (eGFR) ≥50% from the baseline or eGFR <15 ml/min/1.73 m2 or the onset of dialysis).
214 cases of MPGN entered the study with the average age of 44±16 years. Most patients had nephrotic syndrome and significant hematuria. In 58.4% of cases, eGFR was <60 mL/min/1.73 m2, and every fifth patient had CKD 4 or 5 stages. The prevalence of MPGN among all biopsy-confirmed glomerulopathies was 9.3%. Idiopathic MPGN (iMPGN) was detected in 30.4% of cases, secondary MSGN (sMPGN) - in 69.6% (autoimmune diseases - 34.1%, infectious diseases - 16.4%, monoclonal gammopathies - 9.3%, complement-mediated damage - 9.8%). Ig+C3+MPGN was mainly associated with autoimmune diseases and infections; C3-glomerulopathy or thrombotic microangiopathy were most often causes of Ig-C3+MPGN; Ig-C3-/Ig+C3-MPGN had heterogeneous etiology. The median follow-up period was 28 [7; 37] months. The 10-year total cumulative patient and renal survival rates were 71 and 50%, respectively (without differences between sMPGN and iMPGN). The frequency of the PR/CR was 50% (iMPGN - 46.2%, sMPGN - 51.3%) depending on the etiology of the MPGN (p=0.049). The cumulative 10-year progression-free renal survival was nearly 100% in cases with PR/CR and 0% in non-responders.
MPGN is a severe variant of glomerular damage with a heterogeneous etiological structure and an unfavorable prognosis. Targeted clinical and morphological diagnostics of MPGN allows to identify the cause of the disease in most cases. This approach is reliable for the adequate treatment choice and improvement of outcomes in MPGN.
分析膜增生性肾小球肾炎（MPGN）的病因、临床及形态学表现、治疗方法及预后。
回顾性分析2000年至2017年期间的MPGN病例，随后分析其病因、临床资料及形态学（包括免疫球蛋白（Ig）和C3补体成分的沉积）。观察完全缓解和部分缓解（PR，CR）的达成情况、总生存率、疾病进展（复合终点：估计肾小球滤过率（eGFR）较基线下降≥50%或eGFR<15 ml/min/1.73 m2或开始透析）。
214例MPGN患者进入研究，平均年龄44±16岁。大多数患者有肾病综合征和明显血尿。58.4%的病例eGFR<60 mL/min/1.73 m2，每五分之一的患者处于慢性肾脏病4或5期。在所有经活检确诊的肾小球疾病中，MPGN的患病率为9.3%。特发性MPGN（iMPGN）在30.4%的病例中被检测到，继发性MPGN（sMPGN）在69.6%的病例中被检测到（自身免疫性疾病-34.1%，感染性疾病-16.4%，单克隆丙种球蛋白病-9.3%，补体介导的损伤-9.8%）。Ig+C3+MPGN主要与自身免疫性疾病和感染相关；C3肾小球病或血栓性微血管病最常是Ig-C3+MPGN的病因；Ig-C3-/Ig+C3-MPGN病因各异。中位随访期为28[7；37]个月。10年总累积患者生存率和肾脏生存率分别为71%和50%（sMPGN和iMPGN之间无差异）。PR/CR的发生率为50%（iMPGN-46.2%，sMPGN-51.3%），取决于MPGN的病因（p=0.049）。在达到PR/CR的病例中，10年累积无进展肾脏生存率接近100%，在无反应者中为0%。
MPGN是肾小球损伤的一种严重类型，病因结构各异，预后不良。针对性的MPGN临床和形态学诊断在大多数情况下可明确病因。这种方法对于MPGN的合理治疗选择和改善预后是可靠的。
